Extranodal nasal natural killer (NK)/T-cell lymphoma is a very rare kind of lymphoma, Oral cavity involvement of extranodal natural killer/T-cell lymphoma, nasal type is extremely rare, and its clinicopathologic features are also poorly understood. Recently, we experienced an unusual case of Epstein-Barr virus-associated, extranodal NK/T-cell type with a unhealed palatal ulcer as the earliest clinical feature. It is a challenge for oral medicine specialists to make the early diagnosis for this special type of tumor.